ISCHEMICKÉ CMP / KLASIFIKACE A ETIOPATOGENEZE

Created 16/02/2023, last revision 18/02/2023

• celiac disease (gluten enteropathy, endemic sprue, primary malabsorption syndrome) is an immune-mediated systemic disease triggered by gluten in genetically susceptible individuals
  • this response results in characteristic damage to the villi, leading to malabsorption
• it is characterized by a variable combination of clinical symptoms in the presence of specific antibodies, HLA-DQ2 or HLA-DQ8 haplotypes, and enteropathy
• heterogeneous clinical signs and symptoms may begin in either childhood or adult life; disease may be asymptomatic
• celiac disease is associated with an increased incidence of cardiovascular disease including stroke (OR 1.4) [El Moutawakil, 2009]  [Fabbri, 2012]  [Medscape, 2014]
  • stroke may occur without clinical signs of malabsorbtion (both in adulthood and in childhood)
• gluten intolerance is lifelong and incurable, but symptoms resolve with adherence to a gluten-free diet

• celiac disease is associated with an increased risk of venous and arterial thromboembolism (HR for stroke is 1.45) [Poulin, 2015]
  • the mechanisms of vascular involvement in celiac disease are controversial. The most widely incriminated factor is autoimmune central nervous system vasculitis, in which tissue transglutaminase, the main auto-antigen contributing to maintaining the integrity of endothelium tissue, plays a major role. Other mechanisms are still debated, mainly vitamin deficiency

Clinical presentation

• when foods containing gluten are consumed, the lining of the small intestine becomes inflamed and the epithelial cells of the intestine are damaged
• as a result, nutrients may be difficult to absorb and remain undigested in the intestine
• symptoms:
  • weight loss
  • diarrhea
  • vomiting, loss of appetite, fatigue
  • neurological symptoms (epilepsy, bilateral occipital calcification, cerebellar ataxia, degenerative central nervous system disease, peripheric neuropathy, myopathy and stroke)
    • neurological symptoms may occur even without previous signs of malabsorption !!
• celiac disease is associated with an increased risk of:
  • non-Hodgkin’s lymphoma
  • diabetes
  • Hashimoto’s thyroiditis
  • dermatitis herpetiformis Duhring
  • dilated cardiomyopathy  (increased incidence of CD in patients with idiopathic dilated cardiomyopathy as well as in patients with secondary cardiomyopathy has been reported recently)  (Frustaci, 2002)

Diagnostic evaluation

Management

• a gluten-free diet is essential
• thanks to the diet, the intestine heals and the risk of long-term consequences is reduced